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      A Rare Presentation of Conjunctival Myxoma with Pain and Redness: Case Report and Literature Review

      case-report

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          Abstract

          Background

          Conjunctival myxoma is a type of rare, benign tumor of mesenchymal cells, with fewer than 30 reported cases in the English literature. It is mostly an isolated occurrence but can sometimes be associated with systemic diseases such as Carney complex or Zollinger-Ellison syndrome. It is necessary in clinical practice to differentiate it from other similar lesions, such as amelanotic nevus, lymphangioma, myxoid liposarcoma, spindle-cell lipoma, myxoid neurofibroma, and rhabdomyosarcoma.

          Case Presentation

          The usual presentation of conjunctival myxoma is a translucent, well-circumscribed, and painless conjunctival mass, but in this report we discuss an unusual case of conjunctival myxoma in a 47-year-old Taiwanese woman who presented initially with pain and redness. This atypical presentation complicated the diagnosis and the management at first. Surgical excision of the mass was performed. The mass was found to be a conjunctival myxoma. The patient subsequently underwent extensive evaluation but was found not to have any systemic diseases with known association with conjunctival myxoma.

          Conclusions

          In summary, we present a case of conjunctival myxoma in a 47-year-old Taiwanese woman. The initial presentation with pain and redness was atypical for conjunctival myxoma. The lesion was successfully managed with complete excisional biopsy.

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          Most cited references14

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          Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors.

          To report the spectrum and frequency of melanocytic and nonmelanocytic conjunctival tumors in an ocular oncology practice. Retrospective noninterventional case series. One thousand six hundred forty-three consecutive patients with a conjunctival mass evaluated at an ocular oncology department. A chart review was conducted to obtain the clinical features of the patient and tumor and to tabulate and categorize the diagnoses. Tumor diagnosis overall and relative to patient age, race, and gender and relative to tumor location and laterality. In 1643 consecutive patients, the tumor was classified as melanocytic in 872 cases (53%) and nonmelanocytic in 771 cases (47%). The nonmelanocytic categories included congenital choristomatous (n = 40 [2%]), epithelial (n = 219 [13%]), vascular (n = 63 [4%]), fibrous (n = 7 [ 60. Of the 219 patients with epithelial tumors, 80% occurred in males, whereas the incidence of melanocytic lesions was equal in males and females. African-American patients represented only 7% of epithelial tumors, <1% of melanomas, and 8% of lymphoid tumors. Conjunctival tumors were of melanocytic origin in 53% of cases and nonmelanocytic origin in 47%. Overall, melanocytic tumors, epithelial tumors, and lymphoid tumors accounted for 74% of all cases. These tumors were far more common in Caucasian patients, and epithelial tumors were found more frequently in men.
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            Carney complex: Clinical and genetic 2010 update.

            First described in the mid 1980s, Carney complex is a rare dominantly heritable multiple endocrine neoplasia syndrome that affects endocrine glands as the adrenal cortex, the pituitary and the thyroid. It is associated with many other nonendocrine tumors, including cardiac myxomas, testicular tumors, melanotic schwannoma, breast myxomatosis, and abnormal pigmentation or myxomas of the skin. The Carney complex gene 1 was identified 10 years ago as the regulatory subunit 1A of protein kinase A (PRKAR1A) located at 17q22-24. An inactivating heterozygous germ line mutation of PRKAR1A is observed in about two-thirds of Carney complex patients. This last decade many progresses have been done in the knowledge of this rare disease and its genetics. This review outlines the current state of this knowledge on Carney complex. Copyright © 2010 Elsevier Masson SAS. All rights reserved.
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              • Record: found
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              Report of a conjunctival myxoma case and review of the literature.

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                Author and article information

                Journal
                Case Report Ophthalmol
                Case Report Ophthalmol
                COP
                Case Reports in Ophthalmology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                1663-2699
                Jan-Apr 2012
                30 April 2012
                30 April 2012
                : 3
                : 1
                : 145-150
                Affiliations
                [1] aNorthwestern University, Feinberg School of Medicine, Chicago, Ill., USA
                [2] bDepartment of Pathology, St. Mary's Hospital, Taipei Medical University, Taipei, Taiwan, ROC
                [3] cWu-Fu Eye Clinic, Lotung, Taipei Medical University, Taipei, Taiwan, ROC
                [4] dDepartment of Ophthalmology, Taipei Medical University, Taipei, Taiwan, ROC
                Author notes
                *Tommy Yet-Min Lin, MD, DMSC, FACS, No. 108 Chung-Chen Rd., Lotung, I-Lan 265, Taiwan (ROC), Tel. +886 3 954 2416, E-Mail wufu.eye@ 123456msa.hinet.net
                Article
                cop-0003-0145
                10.1159/000338626
                3362239
                22649349
                bc12e53e-fc9a-43f7-beb8-c232c2e4380f
                Copyright © 2012 by S. Karger AG, Basel

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

                History
                Page count
                Figures: 3, Tables: 1, References: 14, Pages: 6
                Categories
                Published online: April, 2012

                Ophthalmology & Optometry
                conjunctival neoplasms, pathology,myxoma, pathology,conjunctival neoplasms, surgery

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